Wilson's Disease

Introduction: Wilson's Disease

Wilson's Disease: Wilson's Disease is a rare autosomal recessive disorder of copper transport, resulting in copper accumulation and toxicity to the liver and brain. (Source: Genes and Disease by the National Center for Biotechnology) ... more about Wilson's Disease.

Wilson's Disease: Genetic disorder that causes copper to build up in the body's organs. More detailed information about the symptoms, causes, and treatments of Wilson's Disease is available below.

Symptoms of Wilson's Disease

• Various neurological and behavioral symptoms - from copper damage to the brain; may be the first symptoms seen in about 60% of cases:
  • Tremor
  • Rigidity of muscles
  • Muscle weakness
  • Muscle paralysis

See full list of 80 symptoms of Wilson's Disease

Medical Textbooks Online about Wilson's Disease

Book excerpts: Copyright © 2008 Lippincott Williams & Wilkins. All rights reserved.

Diagnostic Tests for Wilson's Disease

• Eye exam - for the Kayser-Fleischer ring
• Urine tests
• Urine copper level
• Blood tests
• Blood copper level

See full list of 6 diagnostic tests for Wilson's Disease

Wrongly Diagnosed with Wilson's Disease?

• Misdiagnosis of Wilson's Disease

Misdiagnosis and Wilson's Disease

Rare copper disease insidious and misdiagnosed: Wilson's disease (a form of copper overload) is a rare disorder that has a slow and insidious onset that can often fail to be diagnosed. Copper builds up in the liver...read more »

Huntington disease maybe confused: Huntington Disease is an autosomal dominant disorder presenting as an inherited adult onset neurological disorder. The patient has three characteristic features: chorea, abnormal behaviour and dementia....read more »

Read more about Misdiagnosis and Wilson's Disease

Causes of Wilson's Disease

Read more about causes of Wilson's Disease.

Treatments for Wilson's Disease

• Lifelong treatment
• Low-copper diet - avoid shellfish, chocolate, liver, mushrooms, nuts, and various other foods.
• Vitamin B6 supplements (pyridoxine)
• Zinc supplements
• Anti-copper agents

See full list of 11 treatments for Wilson's Disease

Videos for Wilson's Disease

Patient Surveys for Wilson's Disease

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Prognosis for Wilson's Disease

Prognosis for Wilson's Disease: Good, if treated. Most symptoms are relieved by ongoing treatment and lifespan is normal. Untreated Wilson's disease is usually fatal by 30.

More about prognosis of Wilson's Disease

Reseach about Wilson's Disease

Visit our research pages for current research about Wilson's Disease treatments.

Statistics for Wilson's Disease

• Medical statistics for Wilson's Disease
• Prevalence and incidence statistics for Wilson's Disease
• Death and Mortality statistics for Wilson's Disease
• Society statistics for Wilson's Disease

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Article Excerpts about Wilson's Disease

Genes and Disease by the National Center for Biotechnology (Excerpt)

Wilson's Disease is a rare autosomal recessive disorder of copper transport, resulting in copper accumulation and toxicity to the liver and brain. (Source: Genes and Disease by the National Center for Biotechnology)

Wilson's Disease: NIDDK (Excerpt)

Wilson's disease causes the body to retain copper. The liver of a person who has Wilson's disease does not release copper into bile as it should. Bile is a liquid produced by the liver that helps with digestion. As the intestines absorb copper from food, the copper builds up in the liver and injures liver tissue. Eventually, the damage causes the liver to release the copper directly into the bloodstream, which carries the copper throughout the body. The copper buildup leads to damage in the kidneys, brain, and eyes. If not treated, Wilson's disease can cause severe brain damage, liver failure, and death. (Source: excerpt from Wilson's Disease: NIDDK)

NINDS Wilson's Disease Information Page: NINDS (Excerpt)

Wilson's disease is an inherited disorder in which excessive amounts of copper accumulate in the body. Although the accumulation of copper begins at birth, symptoms of the disorder appear later in life, between the ages of 6 and 40. (Source: excerpt from NINDS Wilson's Disease Information Page: NINDS)

Definitions of Wilson's Disease:

A rare autosomal recessive disease characterized by the deposition of copper in the BRAIN; LIVER; CORNEA; and other organs. It is caused by defects in the ATP7B gene encoding copper-transporting ATPase 2 (EC 3.6.3.4), also known as the Wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as LIVER CIRRHOSIS; TREMOR; ATAXIA and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years. - (Source - Diseases Database)

A rare inherited disorder of copper metabolism; copper accumulates in the liver and then in the red blood cells and brain - (Source - WordNet 2.1)

Wilson's Disease is listed as a "rare disease" by the Office of Rare Diseases (ORD) of the National Institutes of Health (NIH). This means that Wilson's Disease, or a subtype of Wilson's Disease, affects less than 200,000 people in the US population.
Source - National Institutes of Health (NIH)